Original article | Volume 17, Article 55, 25 Jan 2014 | 10.11604/pamj.2014.17.55.3212

The Gorlin-Goltz syndrome: a sporadic case

Kawtar Inani, Fatimazahra Mernissi

Corresponding author: Kawtar Inani, Service de Dermatologie, CHU Hassan II, Fez, Morocco

Received: 11 Aug 2013 - Accepted: 10 Nov 2013 - Published: 25 Jan 2014

Domain: Clinical medicine

Keywords: Gorlin-Goltz syndrome, basal cell nevus syndrome, hereditary disorder

©Kawtar Inani et al. Pan African Medical Journal (ISSN: 1937-8688). This is an Open Access article distributed under the terms of the Creative Commons Attribution International 4.0 License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Cite this article: Kawtar Inani et al. The Gorlin-Goltz syndrome: a sporadic case. Pan African Medical Journal. 2014;17:55. [doi: 10.11604/pamj.2014.17.55.3212]

Available online at: https://www.panafrican-med-journal.com/content/article/17/55/full

Home | Volume 17 | Article number 55

Original article

The Gorlin-Goltz syndrome: a sporadic case

The Gorlin-Goltz syndrome: a sporadic case

 

Kawtar Inani1,&, Fatimazahra Mernissi1

 

1Service de Dermatologie, CHU Hassan II, Fez, Morocco

 

 

&Corresponding author
Kawtar Inani, Service de Dermatologie, CHU Hassan II, Fez, Morocco

 

 

Image in medicine

Gorlin-Goltz syndrome, also known as basal cell nevus syndrome, is an uncommon autosomal dominant hereditary disorder; which is characterized by numerous basal cell carcinomas, maxillary keratocysts and bones malformations. It results from a mutation of the PATCHED gene. The estimate incidence for a general population is 1/50000 to 1/150000. We present a 30 year-old woman, with no similar familial case, who had a repeatedly dental abscesses since the age of 15 years, she consulted for papulonodular lesions that have appeared at the age of eighty. Those lesions were localized on the face and the trunk, reminiscent of basal cell carcinomas. The clinical examination found papulonodular lesions, a mandibular fistula, broadened nasal root, palmer pits and a swelling at the right big toe. The dermoscopic examination of the papulonodular lesions showed a kind of trunk tree arborisation. The biopsy excision of the swelling at the right toe and papulonodulor lesions, revealed respectively an epidermoid cyst and basal cell carcinomas. Radiographic examination showed a calcified falx cerebri calcification, bifid ribs, synostosis, wedged, scoliosis and agenesis of the left kidney. Our patient was treated by a surgical excision of skin lesion with a setting flat of a mandibular fistula. Also a photoprotection was recommended and a regular monitoring of the skin lesions. The Evolution was marked by the emergence of new basal cell carcinomas treated by photodynamic therapy.

Figure 1: A) papulonodular lesions and a broadened nasal root; B) A mandibular fistula; C)MRI image shows a calcified falx cerebri calcification; D) palmer pits

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Original article

The Gorlin-Goltz syndrome: a sporadic case

Original article

The Gorlin-Goltz syndrome: a sporadic case

Original article

The Gorlin-Goltz syndrome: a sporadic case

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Key words

Gorlin-Goltz syndrome

Basal cell nevus syndrome

Hereditary disorder

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The Gorlin-Goltz syndrome: a sporadic case